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A rare hair loss in children: Monilethrix
4297
5462
A rare case of estrogen dermatitis Emilie Bourgeault, MD, Laval University; Janie Bujold, MD, Maria Hospital
A rare hair loss in children: Monilethrix Fernanda Musa Aguiar, MD, Irmandade Santa Casa de Porto Alegre; Leandro Damiani, MD, Irmandade Santa Casa de Porto Alegre; Giselle Martins Pinto, MD, Irmandade Santa Casa de Porto Alegre Monilethrix is a hair shaft disorder characterized by hair fragility and alopecia as a result of hair breakage. It is a rare, hereditary and autosomal dominant genodermatosis, with variable expression. Clinically, the disease may be very characteristic, with extremely fragile and short hair, usually 1-2 cm long, emerging from keratotic follicular papules, especially in the occipital region. As the hair shaft in monilethrix is easily broken, the clinical presentation is of hypertrichosis most pronounced on the occipital scalp, which is more exposed to friction. Trichoscopy is characterized by regular elliptical nodes and intermittent constrictions on the hair shafts, resulting in hair fragility at those points. This is called ‘‘regularly bent ribbon sign.’’ The nodes have a diameter of a normal hair and a medulla, whereas the internodes have no medulla. We report the case of a 7-year-old boy with easily fragmented and short length of hair. Previously, he had never had a haircut. Physical examination showed diffuse hypertrichosis of the scalp, with short, and sparse hairs over the bilateral parietal and occipital region. No sign of scarring of the scalp skin. The diagnosis of monilethrix was confirmed by dermoscopy, which showed regular variations in the diameter of the hair shaft with elliptical nodes and constrictions, giving a beaded appearance to the hair. No similar illness was seen in any family members. Topical minoxidil was began to the patient with mild improve. Monilethrix has inter- and intrafamilial phenotypic variabilities, considering the age of onset, severity, and natural history, ranging from almost normal scalp to total alopecia. The precise mechanisms that cause the periodic change in the diameter of the monilethrix hair are not known yet. An affected newborn will present normal-appearing scalp hair, because the lanugo hair is usually clinically normal and it is progressively replaced by abnormal one during the early months of life. In severe cases the newborn may show a completely bald scalp already at birth and a few remaining hairs are easily ‘‘brushed’’ off. Diagnosis is mostly clinical. The presentation of monilethrix as congenital hypertrichosis is not very common. In this patient, dermoscopy was the clinching tool for diagnosing monilethrix, showing beaded hair. There is no cure for monilethrix. Although there are no specific treatments, topical minoxidil and oral etretinate have all been reported to improve hair growth.
Introduction: Estrogen dermatitis is a rare disorder that is characterized by skin eruptions that recur in a cyclical nature in association with the menstrual cycle. We present an additional case of estrogen dermatitis. Case report: A 43 -year-old woman presented to our dermatology clinic with an 8year history of a recurrent right inguinal pruritic eczematous plaque responsive to a mild topical corticosteroid. Further questioning established that the plaque recurred in a cyclical fashion in association with her menstrual cycle. She had not been exposed to the oral contraceptive pill since her pregnancies 20 years prior, nor was she exposed to any other exogenous forms of estrogen or progesterone. She had no other significant medical history. At the dermatology clinic, patch tests were performed and eliminated an underlying allergic contact dermatitis. Intradermal testing with conjugated estrogen and progesterone were carried out to investigate a possible hormonal relation. The test consisted of 0.5 mL intradermal injections of 5 mg and 0.5 mg concentrations of conjugated estrogen and progesterone, with adequate control. The reading was done at 30 minutes with a positive result for estrogen only. The reaction was more intense with the higher concentration of estrogen. The erythema and induration persisted for at least 12 hours and another reading at 72 hours was negative although the patient described a persistent localized pressure. Discussion: In 1995, Shelley et al coined the term estrogen dermatitis. They described women with premenstrual exacerbations of various eruptions including papulovesicular eruptions, urticaria, eczema and generalized pruritus. Their patients had positive intradermal skin tests to estrogen and responded to tamoxifen therapy. Since the original description, there have been a few reports of the condition with varied treatment approaches including leuprolide acetate progestin only pill and bilateral oophorectomy and hysterectomy. We describe a patient with estrogen dermatitis that presented with mild inguinal eczema. Since her symptoms respond to cyclical application of a mild topical steroid, hormonal treatment was not initiated. Should her symptoms escalate, we could consider treatment with progestin only pill or levonorgestrel intrauterine device as a first-line treatment before more radical treatments. Commercial support: None identified.
Commercial support: None identified.
5166 A rare case of trichodysplasia spinulosa in a patient treated with chemotherapy for acute lymphocytic leukemia Kimya Hassani-Ardakani, BS, McGill University Health Centre; Fatemeh Jafarian, MD, McGill University Health Centre; Van-Hung Nguyen, MD, McGill University Health Centre; Duc-Vinh Thai, MD, McGill University Health Centre Introduction: Trichodysplasia spinulosa (TS) is a rare folliculocentric polyomavirus infection exclusively observed in immunosuppressed patients. TS often presents with clinically distinct spiculated papules and a unique histopathology. Case report: Here we present a 7-year-old boy who was referred to our pediatric dermatology clinic for the evaluation of eruptive papules involving the face. At presentation, the boy was undergoing chemotherapy for acute lymphocytic leukemia (ALL). Interestingly, the patient’s parents recalled the lesions had initially commenced with the initiation of chemotherapy and seemed to be aggravated with the start of each cycle. Physical examination revealed numerous flesh-colored to erythematous papules with central spicules involving the nose and cheeks. Histology demonstrated dilated hair follicles with infundibular keratotic plugging and absent hair shafts. Additionally, the inner root-sheath keratinocytes contained enlarged eosinophilic trichohyalin granules and numerous apoptotic bodies. Based on these clinical and histologic findings, TS was diagnosed. Although the treatment of TS is not well established, our patient and several other cases in the literature have shown clinical improvement with immunosuppressant reduction.
5424 A rare location of pyoderma gangrenosum Alejandra Alfano, MD, Sanatorio G€ uemes; Mariano Marini, MD, Sanatorio G€ uemes; Victoria Campana, MD, Sanatorio G€ uemes; Lorena Mosqueira, MD, Sanatorio G€ uemes; Alejandrina De Prato, MD, Sanatorio G€ uemes; Liliana Iadisernia, MD, Sanatorio G€ uemes; Maria Cavatorta, MD, Sanatorio G€ uemes; Rosana Covielo y Garofalo, MD, Sanatorio G€ uemes; Rocio Lescano, MD, Sanatorio G€ uemes
Discussion and conclusion: Diagnosis of TS is based on identifying characteristic speculated papules and a distinct histopathology. To date, less than 40 cases of TS have been reported, majority of which are associated with organ transplantation. We report the 3rd case of TS in a pediatric patient undergoing chemotherapy for ALL. Given the high seroprevalence of TS-associated polyomavirus, we suspect that TS is under-recognized; therefore, leading to delayed diagnosis and distress for patients and their families. Although rare, TS should be recognized in the differential diagnosis of spiny papules in an immunosuppressed patient.
Pyoderma gangrenosum (PG) is a rare necrotizing skin disease belonging to the group of neutrophilic dermatosis. Several clinical variants have been described. One of them is the ulcerative or classical PG, which is usually located in lower extremities, but also in abdomen, genitalia, head, neck and rarely breast. Primary injury starts as a painful nodule or pustule, either de novo or after minimal trauma. PG often occurs in association with a systemic disease. We are reporting a 51-yearold woman with history of arterial hypertension, attended at gynecology, who presented a painful erythematous nodule in the left breast. She had received several antibiotic treatments with no response and it had progressed to a small painful ulceration, requiring surgical toilette. However, the ulcer increased its size and its margins were surrounded by a halo of erythema. Dermatology department was consulted and a punch biopsy was performed with the diagnosis of ulcerative PG. The patient was treated with high dose of corticoids but no clinical improvement was observed. Minocycline 100 mg once a day was indicated as an alternative therapy during six months with excellent response. The importance of presenting this case is to point out the unusual location of PG in a patient with no previous history of local trauma or systemic disease such as rheumatoid arthritis or inflammatory bowel disease. Although systemic corticosteroids may be useful as a first-line therapy, minocycline has effectively been indicated as an alternative treatment for this neutrophilic dermatosis.
Commercial support: None identified.
Commercial support: None identified.
JUNE 2017
J AM ACAD DERMATOL
AB23
5462
A rare case of estrogen dermatitis Emilie Bourgeault, MD, Laval University; Janie Bujold, MD, Maria Hospital
A rare hair loss in children: Monilethrix Fernanda Musa Aguiar, MD, Irmandade Santa Casa de Porto Alegre; Leandro Damiani, MD, Irmandade Santa Casa de Porto Alegre; Giselle Martins Pinto, MD, Irmandade Santa Casa de Porto Alegre Monilethrix is a hair shaft disorder characterized by hair fragility and alopecia as a result of hair breakage. It is a rare, hereditary and autosomal dominant genodermatosis, with variable expression. Clinically, the disease may be very characteristic, with extremely fragile and short hair, usually 1-2 cm long, emerging from keratotic follicular papules, especially in the occipital region. As the hair shaft in monilethrix is easily broken, the clinical presentation is of hypertrichosis most pronounced on the occipital scalp, which is more exposed to friction. Trichoscopy is characterized by regular elliptical nodes and intermittent constrictions on the hair shafts, resulting in hair fragility at those points. This is called ‘‘regularly bent ribbon sign.’’ The nodes have a diameter of a normal hair and a medulla, whereas the internodes have no medulla. We report the case of a 7-year-old boy with easily fragmented and short length of hair. Previously, he had never had a haircut. Physical examination showed diffuse hypertrichosis of the scalp, with short, and sparse hairs over the bilateral parietal and occipital region. No sign of scarring of the scalp skin. The diagnosis of monilethrix was confirmed by dermoscopy, which showed regular variations in the diameter of the hair shaft with elliptical nodes and constrictions, giving a beaded appearance to the hair. No similar illness was seen in any family members. Topical minoxidil was began to the patient with mild improve. Monilethrix has inter- and intrafamilial phenotypic variabilities, considering the age of onset, severity, and natural history, ranging from almost normal scalp to total alopecia. The precise mechanisms that cause the periodic change in the diameter of the monilethrix hair are not known yet. An affected newborn will present normal-appearing scalp hair, because the lanugo hair is usually clinically normal and it is progressively replaced by abnormal one during the early months of life. In severe cases the newborn may show a completely bald scalp already at birth and a few remaining hairs are easily ‘‘brushed’’ off. Diagnosis is mostly clinical. The presentation of monilethrix as congenital hypertrichosis is not very common. In this patient, dermoscopy was the clinching tool for diagnosing monilethrix, showing beaded hair. There is no cure for monilethrix. Although there are no specific treatments, topical minoxidil and oral etretinate have all been reported to improve hair growth.
Introduction: Estrogen dermatitis is a rare disorder that is characterized by skin eruptions that recur in a cyclical nature in association with the menstrual cycle. We present an additional case of estrogen dermatitis. Case report: A 43 -year-old woman presented to our dermatology clinic with an 8year history of a recurrent right inguinal pruritic eczematous plaque responsive to a mild topical corticosteroid. Further questioning established that the plaque recurred in a cyclical fashion in association with her menstrual cycle. She had not been exposed to the oral contraceptive pill since her pregnancies 20 years prior, nor was she exposed to any other exogenous forms of estrogen or progesterone. She had no other significant medical history. At the dermatology clinic, patch tests were performed and eliminated an underlying allergic contact dermatitis. Intradermal testing with conjugated estrogen and progesterone were carried out to investigate a possible hormonal relation. The test consisted of 0.5 mL intradermal injections of 5 mg and 0.5 mg concentrations of conjugated estrogen and progesterone, with adequate control. The reading was done at 30 minutes with a positive result for estrogen only. The reaction was more intense with the higher concentration of estrogen. The erythema and induration persisted for at least 12 hours and another reading at 72 hours was negative although the patient described a persistent localized pressure. Discussion: In 1995, Shelley et al coined the term estrogen dermatitis. They described women with premenstrual exacerbations of various eruptions including papulovesicular eruptions, urticaria, eczema and generalized pruritus. Their patients had positive intradermal skin tests to estrogen and responded to tamoxifen therapy. Since the original description, there have been a few reports of the condition with varied treatment approaches including leuprolide acetate progestin only pill and bilateral oophorectomy and hysterectomy. We describe a patient with estrogen dermatitis that presented with mild inguinal eczema. Since her symptoms respond to cyclical application of a mild topical steroid, hormonal treatment was not initiated. Should her symptoms escalate, we could consider treatment with progestin only pill or levonorgestrel intrauterine device as a first-line treatment before more radical treatments. Commercial support: None identified.
Commercial support: None identified.
5166 A rare case of trichodysplasia spinulosa in a patient treated with chemotherapy for acute lymphocytic leukemia Kimya Hassani-Ardakani, BS, McGill University Health Centre; Fatemeh Jafarian, MD, McGill University Health Centre; Van-Hung Nguyen, MD, McGill University Health Centre; Duc-Vinh Thai, MD, McGill University Health Centre Introduction: Trichodysplasia spinulosa (TS) is a rare folliculocentric polyomavirus infection exclusively observed in immunosuppressed patients. TS often presents with clinically distinct spiculated papules and a unique histopathology. Case report: Here we present a 7-year-old boy who was referred to our pediatric dermatology clinic for the evaluation of eruptive papules involving the face. At presentation, the boy was undergoing chemotherapy for acute lymphocytic leukemia (ALL). Interestingly, the patient’s parents recalled the lesions had initially commenced with the initiation of chemotherapy and seemed to be aggravated with the start of each cycle. Physical examination revealed numerous flesh-colored to erythematous papules with central spicules involving the nose and cheeks. Histology demonstrated dilated hair follicles with infundibular keratotic plugging and absent hair shafts. Additionally, the inner root-sheath keratinocytes contained enlarged eosinophilic trichohyalin granules and numerous apoptotic bodies. Based on these clinical and histologic findings, TS was diagnosed. Although the treatment of TS is not well established, our patient and several other cases in the literature have shown clinical improvement with immunosuppressant reduction.
5424 A rare location of pyoderma gangrenosum Alejandra Alfano, MD, Sanatorio G€ uemes; Mariano Marini, MD, Sanatorio G€ uemes; Victoria Campana, MD, Sanatorio G€ uemes; Lorena Mosqueira, MD, Sanatorio G€ uemes; Alejandrina De Prato, MD, Sanatorio G€ uemes; Liliana Iadisernia, MD, Sanatorio G€ uemes; Maria Cavatorta, MD, Sanatorio G€ uemes; Rosana Covielo y Garofalo, MD, Sanatorio G€ uemes; Rocio Lescano, MD, Sanatorio G€ uemes
Discussion and conclusion: Diagnosis of TS is based on identifying characteristic speculated papules and a distinct histopathology. To date, less than 40 cases of TS have been reported, majority of which are associated with organ transplantation. We report the 3rd case of TS in a pediatric patient undergoing chemotherapy for ALL. Given the high seroprevalence of TS-associated polyomavirus, we suspect that TS is under-recognized; therefore, leading to delayed diagnosis and distress for patients and their families. Although rare, TS should be recognized in the differential diagnosis of spiny papules in an immunosuppressed patient.
Pyoderma gangrenosum (PG) is a rare necrotizing skin disease belonging to the group of neutrophilic dermatosis. Several clinical variants have been described. One of them is the ulcerative or classical PG, which is usually located in lower extremities, but also in abdomen, genitalia, head, neck and rarely breast. Primary injury starts as a painful nodule or pustule, either de novo or after minimal trauma. PG often occurs in association with a systemic disease. We are reporting a 51-yearold woman with history of arterial hypertension, attended at gynecology, who presented a painful erythematous nodule in the left breast. She had received several antibiotic treatments with no response and it had progressed to a small painful ulceration, requiring surgical toilette. However, the ulcer increased its size and its margins were surrounded by a halo of erythema. Dermatology department was consulted and a punch biopsy was performed with the diagnosis of ulcerative PG. The patient was treated with high dose of corticoids but no clinical improvement was observed. Minocycline 100 mg once a day was indicated as an alternative therapy during six months with excellent response. The importance of presenting this case is to point out the unusual location of PG in a patient with no previous history of local trauma or systemic disease such as rheumatoid arthritis or inflammatory bowel disease. Although systemic corticosteroids may be useful as a first-line therapy, minocycline has effectively been indicated as an alternative treatment for this neutrophilic dermatosis.
Commercial support: None identified.
Commercial support: None identified.
JUNE 2017
J AM ACAD DERMATOL
AB23