diffuse partial wooly hair ( dpwh ) was originally reported by ormerod et al . in a family of six individuals . it is characterized by fine , short , and kinky hairs interspersed with normal hair throughout the scalp . the proportion of abnormal hairs has been calculated to amount from 20% to 30% of hairs . the possibility that dpwh may be associated with progressive miniaturization of hair follicles may account for the presence of hair thinning in adult patients affected by this abnormality . we report a case of dpwh in a patient with epidermolysis bullosa simplex with mottled pigmentation ( ebs - mt ) , and discuss whether this association is fortuitous or whether dpwh may have an as yet unidentified underlying keratin disorder in common with ebs - mt . a 35-year - old female patient presented with a history of unruly hair and the complaint of hair thinning . clinical examination revealed coarse hair and an irregular central hair part line with small clear areas on both sides [ figure 1a ] . closer examination exhibited two types of hair shafts : the first were straight , while the second were wavy and shorter [ figure 2 ] . the second type of hair was diffusely interspersed with the normal hair throughout the scalp . the proportion of wavy hairs in a probe of 212 hairs obtained by cutting was calculated to be 28% . ( a ) coarse hair and an irregular central hair part line with small clear areas on both sides . ( b ) coarse hair and tightened central hair part line two populations of hair shafts : normal hair shafts are straight , the second type of hair shafts are wavy and shorter examination of the skin showed discrete pigmented spots located on the extremities and punctate palmar keratoses . the patient was otherwise healthy , and was the first in her family with ebs - mt . she reported that her mother had alopecia , not otherwise specified , and that her two daughters suffered from the same skin problems . based on the clinical findings a diagnosis of dpwh in combination with female androgenetic alopecia in a patient with ebs - mt was established . treatment with 5% topical minoxidil solution once daily in combination with 5 mg oral biotin was prescribed , and the patient was advised to use a shampoo designed for care of dry and unruly hair on the basis of papyrus milk and cationic polymers . at 6-month follow - up of treatment , the widened central hair part tightened , and dermoscopy revealed significant reduction of anisotrichosis , while the proportion of wavy hairs remained unchanged [ figure 1b ] . nevertheless , the patient reported improvement of her hair condition in terms of density and manageability , and was overall satisfied with the treatment outcome . according to the literature data so far there have been single reports and small case series regarding dpwh . our patient presented the typical features of the disorder with first symptoms appearing during adolescence , hair dysplasia limited to the scalp hair and two distinct populations of hair shafts scattered over the entire scalp with a proportion of woolly hair of approximately 20 - 30% . previously others have additionally drawn attention to the complaint of hair thinning . while lalevic - vasic et al . interpreted this finding as due to a progressive loss of wavy hairs , guidetti et al . proposed that dpwh may result from a progressive miniaturization of the hair follicles . in the first case , the differential diagnosis would have to be made from partial diffuse woolly hair associated with the loose anagen hair syndrome , and in the latter from acquired kinking of the hair in androgenetic alopecia . our case fulfills the criteria for diagnosis of dpwh , and we interpret androgenetic alopecia to represent a co - morbidity . oral biotin was given , besides special hair care recommendations , with the aim to improve the condition of the hair shaft , as previously reported to be beneficial in siblings with ectodermal dysplasia and uncombable hair . contrary to other entities within the woolly hair group , dpwh so far has not been associated with any other disorder , particularly palmoplantar keratosis and cardiac anomalies . in our patient , ebs - mt represents an autosomal dominant disorder that is characterized by nonscaring blister formation usually on palms and soles at birth or in infancy in association with abnormal skin pigmentation later in life . the gene mutation responsible for ebs - mt has been identified to affect the keratin 5 gene . while woolly hair ( in association with hypotrichosis ) has been described in association with the ebs skin fragility syndromes due to mutations of desmoplakin ( skin fragility - woolly hair syndrome ) , plakophilin 1 ( skin fragility - ectodermal dysplasia syndrome ) , or plakoglobin ( skin fragility plakoglobin deficiency ) , dpwh has not been previously reported in ebs - mt . this association may , therefore , be fortuitous , or dpwh may have an as yet unidentified underlying keratin disorder in common with ebs - mt . further observations and studies into the molecular and genetic basis of dpwh are required to confirm or exclude the possible connection .
diffuse partial woolly hair ( dpwh ) is an uncommon pilar dysplasia defined by the presence of two hair shaft populations with wooly hairs distributed diffusely among normal hairs throughout the scalp . 
 so far the condition has been reported as an isolated disorder with familial occurrence . 
 we report a case of dpwh in 35-year - old female patient with epidermolysis bullosa with mottled pigmentation .