Hair breakage as a presenting sign of early or occult central centrifugal cicatricial alopecia: clinicopathologic findings in 9 patients.

BACKGROUND: Central centrifugal cicatricial alopecia is the most common form of cicatricial alopecia in African American women. Treatment options are limited and mostly aimed at halting further hair loss but rarely result in hair regrowth. Therefore, it is important to recognize early clinical signs, perform a confirmatory biopsy, and begin treatment promptly. We have observed that hair breakage may be a key sign of early central centrifugal cicatricial alopecia, and this association is not clearly described in the literature. OBSERVATIONS: Nine patients with hair breakage on the vertex with or without scalp symptoms underwent scalp biopsies as part of their evaluation. Of these, 8 had histologic samples adequate for complete interpretation: 5 specimens (63%) showed histologic changes typical of central centrifugal cicatricial alopecia, with 1 of these showing advanced end-stage changes of cicatricial alopecia. Two (25%) revealed premature desquamation of the inner root sheath as the sole finding suggestive of early central centrifugal cicatricial alopecia and 1 (13%) was normal. CONCLUSIONS: Although hair breakage can have multiple causes, early central centrifugal cicatricial alopecia must be considered in the differential diagnosis, particularly in women of African ancestry. Histologic evaluation may reveal early or late findings that can help establish the diagnosis.

Histopathologic study of hidradenitis suppurativa following long-pulsed 1064-nm Nd:YAG laser treatment.

OBJECTIVE: To assess clinical and histopathologic changes occurring after long-pulsed 1064-nm Nd:YAG laser treatment of hidradenitis suppurativa (HS). DESIGN: Prospective, controlled clinical and histologic study of patients with Hurley stage II HS disease. SETTING: Outpatient dermatology department at Henry Ford Hospital, Detroit, Michigan. PARTICIPANTS: Nineteen patients with Fitzpatrick skin types II to VI with Hurley stage II HS lesions of the axilla and groin. Interventions Two monthly laser sessions were performed using the long-pulsed 1064-nm Nd:YAG laser. Main Outcome Measure Clinical response was scored using the modified Sartorius scale for HS reflecting Lesion Area and Severity Index (LASI). Histologic changes were examined before treatment and 1 week, 1 month, and 2 months after treatment. RESULTS: The percentage change in HS severity after 2 sessions of laser treatment was -31.6 over all anatomic sites (P < .005), -24.4 for the axillary site (P = .008), and -36.8 for the inguinal site (P = .001). Histologic changes corresponded to clinical response. Findings from serial biopsy specimens showed increased inflammation at 1 week after treatment and decreased inflammation with resulting fibrosis and scarring at 1 month and 2 months after treatment. CONCLUSIONS: The long-pulsed 1064-nm Nd:YAG laser is a novel effective treatment option for HS. Our histopathologic data suggest that HS is primarily a follicular disorder. The Nd:YAG laser penetrates for selective photothermolysis of the follicular unit and destruction of organized inflammatory lesions in the superficial to mid dermis. Our study offers insight into the pathogenesis of HS and the mechanism of the Nd:YAG laser in treatment of patients with this chronic, debilitating disease.

The misnomer "macrocephaly-cutis marmorata telangiectatica congenita syndrome": report of 12 new cases and support for revising the name to macrocephaly-capillary malformations.

BACKGROUND: The condition known as macrocephaly-cutis marmorata telangiectatica congenita syndrome (M-CMTC) is a rare congenital syndrome of unknown etiology characterized by macrocephaly and vascular lesions that have been described as either cutis marmorata or cutis marmorata telangiectatica congenita (CMTC). Most patients also exhibit facial and limb asymmetry; somatic overgrowth; developmental delay; capillary malformations of the nose, philtrum, and/or upper lip; neurologic abnormalities; syndactyly or polydactyly; craniofacial abnormalities; and joint laxity or soft skin. OBSERVATIONS: We describe 12 patients with this condition from tertiary care medical centers (8 cases) and accrued via an M-CMTC support group Web site (4 cases). All patients showed reticulated or confluent port-wine stains (PWS), not CMTC. Seven of the 12 patients also had centrofacial capillary malformations. In our comprehensive review of 100 previously reported cases, only 34 were accompanied by photographs that were sufficiently clear to review for diagnostic purposes. None had true CMTC, with most having reticulated PWS or persistent cutis marmorata. CONCLUSIONS: Reticulated or confluent PWS and persistent capillary malformations of the central face, rather than CMTC, are the most characteristic cutaneous vascular anomalies seen in so-called M-CMTC syndrome. The name macrocephaly-capillary malformations (M-CM) more accurately reflects the features of this syndrome.